This paper documents activation of an unfolded protein response (UPR) in oligodendrocytes in mouse models of Pelizaeus-Mezbacher disease (PMD) and goes on to show that mice lacking the Chop gene have a much more severe course of what is otherwise a mild disease associated with the rumpshaker (rsh) mutation that causes PMD.

This last observation is the highlight of the paper as it implicates CHOP, a gene that functions in the...

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